Author + information
- Received December 20, 2018
- Revision received February 2, 2019
- Accepted February 3, 2019
- Published online March 18, 2019.
- Zachary Laksman, MD, MSc∗ (, )
- Scott Barichello, MD,
- Thomas M. Roston, MD,
- Marc W. Deyell, MD, MSc and
- Andrew D. Krahn, MD
- Heart Rhythm Services, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
- ↵∗Address for correspondence:
Dr. Zachary Laksman, Heart Rhythm Vancouver, 211-1033 Davie Street, Vancouver, British Columbia V6E 1M7, Canada.
• Ventricular arrhythmias in a patient without structural or ischemic heart disease should raise suspicion for the presence of an underlying inherited heart rhythm disorder.
• Employment of ACLS protocols in patients with inherited arrhythmia syndromes may actually promote arrhythmia; therefore, it is essential that clinicians have an expeditious and reliable approach for identifying the specific etiology and providing tailored therapy.
• This review focuses on the analysis of the patient’s baseline ECG and mode of initiation of the presenting arrhythmia to aid in the bedside management of a patient with a suspected underlying inherited arrhythmia syndrome based on currently available evidence.
• Where clinical experience and best practice guidelines are lacking, large ongoing registries and well designed clinical trials are required to bridge the knowledge gap that remains between our understanding of pathophysiologic mechanisms and clinical outcomes.
After the most common causes of sudden cardiac death including ischemic and structural heart disease have been ruled out, clinicians on the front lines of emergent medical care can be faced with unexplained and recurrent life-threatening arrhythmia episodes in children and adults. In these cases, an inherited arrhythmia syndrome should be suspected, and a departure from conventional advanced cardiac life support algorithms may be required. This review focuses on the electrocardiographic clues of an inherited arrhythmia syndrome that can be uncovered through a careful analysis of the baseline electrocardiogram (ECG) and classification of the presenting ventricular arrhythmia and its mode of onset. After presenting an informed working diagnosis and an explanation of the implied electrophysiologic mechanisms, discussion provides a protocol approach to acute and subacute management decisions. Careful attention to a patient’s response to treatment and its ECG surrogates have the potential to facilitate tailored therapy based on the underlying arrhythmogenic substrate and pathophysiology.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
All authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received December 20, 2018.
- Revision received February 2, 2019.
- Accepted February 3, 2019.
- 2019 American College of Cardiology Foundation
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